By Malerie Hartsell, MPH, CHES
Children with Special Health Care Needs
September is National Sickle Cell Awareness Month. The purpose of the month is to bring attention to a crippling genetic disease that many people around the world struggle with and manage daily.
Sickle Cell Awareness Month helps to debunk stereotypes and stigmas associated with persons who have sickle cell disease. Not only does this month shine light on sickle cell disease (SCD) but also sickle cell trait (SCT).
Here are Key Points to know about SCD & SCT:
- SCD is a blood disorder that causes sickling of the red blood cells.
- SCD is the most common hereditary blood disorder.
- Red blood cells become hard and sticky and look like a C-shaped farm tool called a “sickle”.
- Persons who have SCD may suffer from crises — acute episodes of intense and excruciating pain.
- Different types of sickle cell disease are caused by different genetic mutations.
- SCT is where a person inherits one sickle cell gene and one normal gene from either of their parents.
- Persons with SCT can pass the trait on to their children.
Who is affected?
While sickle cell disease affects millions of people throughout the world, it is more common among African-Americans. It is estimated that SCD affects 100,000 people in the United States.
Sickle cell trait is estimated to affect 1 million to 3 million Americans and 8 to 10 percent of African-Americans. Sickle cell trait can also affect Hispanics, South Asians, Caucasians from southern Europe, and people from Middle Eastern countries. More than 100 million people worldwide have sickle cell trait.
While SCD is inherited when a child receives two sickle cell genes from both parents, for someone that has SCT, the likelihood of having a child that has SCD or SCT is different. If both parents have SCT, there is a 50 percent chance the child will have SCT, a 25 percent chance the child may have SCD, and a 25 percent chance the child will have not have SCD or SCT.
Care and Treatment
Early diagnosis and monitoring can make a difference in the quality of life and number of years lived for someone with SCD. The Center for Disease and Control recommends:
- Babies from birth to 1 year of age should see a doctor every two to three months.
- Children from 1 to 2 years of age should see a doctor at least every three months.
- Children and adults 2 years of age or older should see a doctor at least once every year.
Persons with SCD should be referred to a hematologist or an experienced general pediatrician, internist or family practitioner.
There are treatments that can help to prevent complications and medicate the acute or chronic pain episodes as they occur. Treatment options for persons with SCD differ by patient. Children with SCD can begin taking daily doses of penicillin starting at birth up to 5 years to help prevent bacterial infections. Blood transfusions are utilized to help reduce the risk of stroke. Hydroxyurea, a drug that increases levels of fetal hemoglobin that is less likely to become sickle-shaped, has helped reduce pain, hospitalizations and lung damage.
Currently hematopoietic stem cell transplantation (HSCT) is the only cure for SCD.
For more information about sickle cell, visit http://www.cdc.gov/ncbddd/sicklecell/index.html or http://www.nhlbi.nih.gov/health/health-topics/topics/sca.